Researchers have identified distinctive clinical signs of acute sleepy myelitis in polio-like diseases (AFM), shows a new report. They propose…
Researchers have identified distinctive clinical signs of acute sleepy myelitis in polio-like diseases (AFM), shows a new report. They propose a more restrictive definition of the disease to help identify the cases faster and to distinguish such cases from other diseases that can cause acute weakness.
The current definition of Centers for Disease Control and Prevention (CDC) was designed to capture all possible cases of epidemiological monitoring of the emerging disease, which was first recognized in 2014. However, the definition is so wide that some cases of disease will be misdiagnosed as AFM.
In the present case series of 45 patients, 24% were found to have a definable alternative diagnosis, mainly transverse myelitis and spinal cord reaction. Other alternative diagnoses included demyelinating syndrome, Guillain-Barré syndrome, Chiari I myelopathy and meningitis.
Matthew J. Elrick, MD, PhD student at the Neurological Department at Johns Hopkins University’s School of Medicine in Baltimore, Maryland, and colleagues report their findings in an article published online on November 30 in JAMA Pediatrics.
Elrick and colleagues studied 45 patients under the age of 1
8 years diagnosed on the basis of CDC criteria as AFM between 2012 and 2016. The investigators divided the patients into two groups. Group 1 included patients from the United States and Canada, whose condition met the CDC definition of AFM. Group 2 included patients referring to Johns Hopkins Transverse Myelitis Center for Evaluation of Suspect AFM.
Patient records and formation data were reviewed by three neurologists to identify patients for whom there were possible alternative diagnoses. The remaining patients were considered to have AFM on the basis of a more restrictive definition (RAFM).
The researchers then compared the clinical characteristics of those with rAFM to those with other diagnoses to develop a case description for RAFM.
The restrictive definition requires that the following parts are present:
Occurrence of fever or virus syndrome.
Weakness in a lower engine neuron pattern involving one or more limbs, neck, face and / or bulb muscles. In addition, weak limbs should have less muscle tone and late reflections should be reduced or missing.
At least one of the following: Gravity dominant T2 hyperintension injury on the spinal cord of the spinal cord that spans many levels, with or without enhancement of ventral nerve root Evidence from electromyography and neuronal study of a motor neuronopathy with intact sensory nerve conduction. or cerebrospinal fluid (CSF) studies indicating pleocytosis (number of white blood cells> 5 cells / μl).
In addition, the definition specifies that the following factors or characteristics should be missing:
Supratentorial white material or cortical damage> 1 cm
Encephalopathy, which can not be explained by fever, disease, respiratory distress or metabolic abnormalities  Increase of CSF protein greater than twice the upper limit of normal in the absence of CSF pleocytosis  A definable alternative diagnosis
The authors emphasize that the restrictive definition “is deliberately designed with high specificity as a target and acknowledges that sensitivity will be less than that of the CDC case definition. “
The narrower definition is likely to be most useful for research by enabling better identification of pathogens and potential treatments and assisting with differential diagnosis, although more research is needed to confirm the results.
AFM seems to occur in waves every two years, the authors say. Next wave is expected in 2020.
“Clinics should be prepared to recognize cases presented in the late summer and fall, often after viral respiratory infection, and present with asymmetric weakness that develops over hours to days associated with characteristic MRI and CSF findings “, the authors write.
As recently reported by Medscape Medical News, from November 13, 90 CDC-confirmed cases of AFM had been reported in 27 states by 2018.  Keys of Confirmation
“The Leaders The diagnosis lies in the patient’s history and physical examination, and the keys to confirmation come from lumbar spine and magnetic resonance formation (MRI) in the spinal cord, “writes Sarah E. Hopkins, MD, MSPH, from the Division of Neurology at Children‘s Hospital in Philadelphia , Pennsylvania, and colleagues in an accompanying editorial.
When clinics suspect AFM, hospitalization is recommended as they write because the patient can weaken quickly. Particularly in the risk zone, patients with upper limbs and bulbs are weakness; These patients may need to be taken into a childcare unit.
“All patients need respiratory monitoring with actions like negative inspirational power and forced vital capacity. Constipation is prominent and aggressive handling is motivated,” adds. 19659022] They note that AFM can involve asymmetric areas and mainly proximal muscles. In presentation, the patient’s character can range from barely noticeable weakness in an extremity to quadriparesis requiring respiratory support and a supply tube.
“Spinal cord-related stroke should be considered in case of vascular risk factors, recent trauma or hyperacute onset,” write Hopkins and colleagues.
In Guillain-Barré syndrome, the pattern of weakness differs. Normally, distal muscles are affected, rather than proximal muscles, with symmetrically increasing progression, the authors write.
With AFM, extremity pain can be extreme, and treatment of neuropathic pain is helpful, says editorial staff.
study authors and editors stress the need to quickly collect samples. CSF should be assessed and nasopharyngeal and orofaryngeal swabs, serum and stool or rectal swabs should be taken. “Quick trial purchase with copy copy and data to CDC will maximize the identification of associated infections and help determine the next step for prevention, including vaccine development,” the editors write.
The study was partially financed by the Johns Hopkins University Provost Catalyst Award, supported by Bart McLean Fund for Neuroimmunology Research and Project Restore. The relevant economic relations of the authors and editors are listed in the original articles.
JAMA Pediatrician. Published online on November 30, 2018. Full text, editorial